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For those of you who have children who have had a liver biopsy, I'd love to hear

more about the process. Can you get a mito. diagnosis through a liver biopsy

just like a muscle biopsy?

We are considering this for Carsen since his liver is abnormal.

Thanks for any information.

Krystena s

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> Krystena s,

actually had a normal muscle and skin fibroblast biopsy.

It was her liver that the poor mito function was found in. They are

thinking when they biopsy me (probably soon) that they may skip the

others and go right to the liver. Originally she had about 3 inches

of liver removed in an open surgery, but recently she had a

laparoscope done with three core biopsies taken. Open surgery was a

little longer recovery but really not to bad. This time she had a

liver needle, mediport replaced, bone marrow biopsy and skin

fibroblast and she was up and better in 2 days!

I think the biggest concern with any surgery is the anesthesia. We

had a great well versed anesthesiologist who took every precaution

and all went great! Hope this helps a little.

Dawn

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> Krystena s,

actually had a normal muscle and skin fibroblast biopsy.

It was her liver that the poor mito function was found in. They are

thinking when they biopsy me (probably soon) that they may skip the

others and go right to the liver. Originally she had about 3 inches

of liver removed in an open surgery, but recently she had a

laparoscope done with three core biopsies taken. Open surgery was a

little longer recovery but really not to bad. This time she had a

liver needle, mediport replaced, bone marrow biopsy and skin

fibroblast and she was up and better in 2 days!

I think the biggest concern with any surgery is the anesthesia. We

had a great well versed anesthesiologist who took every precaution

and all went great! Hope this helps a little.

Dawn

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  • 5 years later...
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Hi Danny;

Because your bilirubin and alkaline phosphatase have responded to prednisone, it

might be worth asking your Dr. if it would be worth testing for elevated IgG4.

There is a new type of sclerosing cholangitis now being described that resembles

autoimmune pancreatitis, is associated with elevated IgG4, and responds to

corticosteroids:

________________________

Best Pract Res Clin Gastroenterol. 2009;23(1):11-23.

Autoimmune pancreatitis and IgG4-associated sclerosing cholangitis.

Nishimori I, Otsuki M.

Department of Gastroenterology and Hepatology, Kochi Medical School, Nankoku,

Kochi 783-8505, Japan. nisao@...

Autoimmune pancreatitis (AIP) is a unique form of chronic pancreatitis

characterised by a high serum IgG4 concentration and complications that include

various extrapancreatic manifestations, one of which is sclerosing cholangitis.

In AIP patients, infiltration of abundant IgG4-positive plasma cells and dense

fibrosis are commonly observed in the pancreas and wall of the bile duct and

gallbladder. The major symptom at onset of AIP is obstructive jaundice caused by

stricture of the bile duct, and this requires differential diagnosis of AIP from

pancreato-biliary malignancies and primary sclerosing cholangitis (PSC).

Recently, there have been reports of particular cases of sclerosing cholangitis

with a high serum IgG4 level and cholangiographic and pathological findings

comparable to those observed in AIP patients. Being apparently different from

PSC and similar to that in AIP, sclerosing cholangitis with and without AIP

shows a clinical response to steroid therapy and thus is designated as

'IgG4-associated sclerosing cholangitis'. The pathogenesis of AIP and

IgG4-associated sclerosing cholangitis remains at yet undetermined.

___________________________________

Am J Gastroenterol. 2006 Sep;101(9):2070-5.

Elevated serum IgG4 concentration in patients with primary sclerosing

cholangitis.

Mendes FD, nsen R, Keach J, Katzmann JA, Smyrk T, Donlinger J, Chari S,

Lindor KD

Gastroenterology and Hepatology, Mayo Clinic College of Medicine, Rochester,

Minnesota 55905, USA

OBJECTIVES: Biliary strictures, similar to primary sclerosing cholangitis (PSC),

have been reported in patients with autoimmune pancreatitis, which is

characterized by elevated serum IgG4 levels and responsiveness to

corticosteroids. We sought to determine the frequency of elevated IgG4 in

patients with PSC and to clinically compare PSC patients with elevated and

normal IgG4 levels. METHODS: We measured serum IgG4 in 127 patients with PSC and

87 patients with primary biliary cirrhosis, as disease controls. Demographic,

clinical, and laboratory characteristics were compared between the PSC groups

with normal and elevated IgG4 (>140 mg/dL). RESULTS: Elevated IgG4 was found in

12 PSC patients (9%) versus one PBC patient (1.1%) (p= 0.017). Patients with

elevated IgG4 had higher total bilirubin (p= 0.009), alkaline phosphatase (p=

0.01), and PSC Mayo risk score (p= 0.038), and lower frequency of IBD (p <

0.0001). Importantly, the time to liver transplantation was shorter in patients

with elevated IgG4 (1.7 vs 6.5 yr, p= 0.0009). The type of biliary involvement

(intrahepatic, extrahepatic, or both) and pancreatic involvement were similar in

both groups. CONCLUSIONS: A small proportion of PSC patients had elevated serum

IgG4. In these patients parameters of liver disease severity were more

pronounced and time to liver transplantation was shorter, suggesting a more

severe disease course. It is possible that this subset of patients behaves

similarly to autoimmune pancreatitis patients with biliary strictures, and could

potentially respond to corticosteroids. Testing PSC patients for IgG4 and

treating those with elevated levels with corticosteroids in clinical trials

should be considered. PMID: 16879434

_____________________________

A biopsy might tell whether you have autoimmune hepatitis and/or PSC, but I'm

really not sure how the course of prednisone prior to the biopsy might affect

the results.

I'm not sure what an ERCP would accomplish at this point if the MRCP gave good

images?

Best regards,

Dave

(father of (23); PSC 07/03; UC 08/03)

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